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B淋巴细胞的原发性免疫缺陷

Primary immunodeficiencies of the B lymphocyte.

作者信息

Moise Ana, Nedelcu Filofteia Daniela, Toader Maria Adela, Sora Steluta Mihaela, Tica Anca, Ferastraoaru Denisa Elena, Constantinescu Ileana

机构信息

Transplant Immunology Department, Carol Davila University of Medicine and Farmacy, Bucharest, Romania.

出版信息

J Med Life. 2010 Jan-Mar;3(1):60-3.

Abstract

The immune response consists of two main components: humoral immunity represented by B lymphocytes and cellular immunity maintained by the T lymphocytes. Immunoglobulins, produced by B-lymphocytes, are the main mediators of humoral immunity, and deficiencies at this level affect the body's response to infection. Plasmocytes produce nine antibody izotypes: immunoglobulins G (IgG1, IgG2, IgG3, IgG4), immunoglobulins M (IgM), immunoglobulins A (IgA1, IgA2), immunoglobulins D (IGD) and immunoglobulins E (IgE). Primary hypogammaglobulinemias are characterized by the occurrence of recurrent infections and, paradoxically, by the occurrence of autoimmune diseases. Characteristic for these diseases is that symptoms occur at 7-9 months after birth, when transplacental antibody titers transmitted from the mother decrease, and the infant's body is unable to synthesize them to normal levels. Primary hypogammaglobulinemias are transmitted genetically, but mutations at the molecular level are still not fully understood. The most common are: Bruton agammaglobulinemia, transient newborn hypogammaglobulinemia, selective immunoglobulin deficiency and variable common immunodeficiency. Treatment consists of monthly antibiotics and immunoglobulins, depending on antibody titers (except for IgA deficiency).

摘要

免疫反应由两个主要成分组成

以B淋巴细胞为代表的体液免疫和由T淋巴细胞维持的细胞免疫。B淋巴细胞产生的免疫球蛋白是体液免疫的主要介质,这一水平的缺陷会影响机体对感染的反应。浆细胞产生九种抗体同种型:免疫球蛋白G(IgG1、IgG2、IgG3、IgG4)、免疫球蛋白M(IgM)、免疫球蛋白A(IgA1、IgA2)、免疫球蛋白D(IGD)和免疫球蛋白E(IgE)。原发性低丙种球蛋白血症的特征是反复感染的发生,且矛盾的是,还会出现自身免疫性疾病。这些疾病的特点是症状在出生后7至9个月出现,此时从母亲传递的经胎盘抗体滴度下降,而婴儿身体无法将它们合成到正常水平。原发性低丙种球蛋白血症是遗传性的,但分子水平的突变仍未完全了解。最常见的有:布鲁顿无丙种球蛋白血症、新生儿短暂性低丙种球蛋白血症、选择性免疫球蛋白缺乏症和可变常见免疫缺陷。治疗包括根据抗体滴度每月使用抗生素和免疫球蛋白(IgA缺乏症除外)。

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